骨纤维性结构不良为良性特发性疾病。首由Lichtenstein等(1942)描述,少数有肉瘤样改变,有时伴有内分泌障碍及皮肤与粘膜色素改变。有单骨型(70％)及多骨型(又可分为不伴骨外病变与伴骨外病变者,后者如McCune-Albright综合征,甚少见)。颞骨发病很少见,但以单骨型为常见。本文报告1例12岁男孩,诉7个月来进行性听力减低,外耳道被骨性肿胀堵塞,拟诊骨瘤。3个月后颞骨鳞部及乳突部肿胀,乳 Poor bone fibrous structure is a benign idiopathic disease. First described by Lichtenstein et al (1942), a few have sarcomatoid changes, sometimes accompanied by endocrine disorders and skin and mucosal pigment changes. There are a single bone type (70%) and bony type (which can be divided into non-ectopic and ectopic lesions, the latter such as McCune-Albright syndrome, rare). Temporal bone disease is rare, but the single bone type is common. This article reports a 12-year-old boy, v. 7 months of progressive hearing loss, external auditory meatal blockage of osseous swelling, diagnosis of osteoma. 3 months after the temporal bone squamous and mastoid swelling, milk